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Diagnosis and staging of Rasmussen’s encephalitis by serial MRI and histopathology

From the Departments of Epileptology (Drs. Bien and Elger), Radiology (Dr. Urbach), Neuropathology (Drs. Bien, Deckert, and Wiestler), and Neurosurgery (Dr. Schramm), University of Bonn, Germany; and Brain Research Institute (Dr. Lassmann), Department of Neuroimmunology, University of Vienna, Austria.

Address correspondence and reprint requests to Dr. C. Bien, University of Bonn, Department of Epileptology, Sigmund-Freud-Str. 25, D-53105 Bonn, Germany; e-mail: c.bien{at}uni-bonn.de

Objective: To correlate MRI and histopathologic findings in patients with Rasmussen’s encephalitis (RE).

Patients and methods: MRI features of 10 patients with RE were studied on serial scans. In surgical specimens from these patients, densities of T lymphocytes, microglial cells and nodules, and reactive astrocytes were evaluated.

Results: Densities of T cells, microglial nodules, and astrocytes were inversely correlated to disease duration. MRI abnormalities had a focal onset and spread across one hemisphere. The following course of MRI abnormalities in a given brain region was observed: on very early scans, the cortex was swollen and showed a hyperintense T2/fluid-attenuated inversion recovery signal. Consecutively, progressive atrophy of the affected hemisphere occurred. Correlation of MRI features with quantitative histopathology revealed that there was a higher number of T cells and reactive astrocytes in the earlier MRI stages compared with the late (merely atrophic) stage.

Conclusion: These data suggest a disease course in RE with the highest inflammatory intensity in the early stages and a subsequent decrease in inflammation. The MRI abnormalities and their characteristic sequence may help to identify patients with RE and to obtain informative biopsies.

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